Glomerulonephritis with Non-Randall-type, Non-Cryoglobulinaemic Monoclonal Immunoglobulin G Deposits (PGNMID and ITG). - Université de Picardie Jules Verne Accéder directement au contenu
Article Dans Une Revue Clinical Kidney Journal Année : 2022

Glomerulonephritis with Non-Randall-type, Non-Cryoglobulinaemic Monoclonal Immunoglobulin G Deposits (PGNMID and ITG).

1 UPJV - Université de Picardie Jules Verne
2 CHU Amiens-Picardie
3 Centre hospitalier [Valenciennes, Nord]
4 PARCC (UMR_S 970/ U970) - Paris-Centre de Recherche Cardiovasculaire
5 HEGP - Hôpital Européen Georges Pompidou [APHP]
6 Nouvel Hôpital Civil de Strasbourg
7 CoRaKID - Maladies rénales fréquentes et rares : des mécanismes moléculaires à la médecine personnalisée
8 CHU Tenon [AP-HP]
9 Hôpital Henri Mondor
10 EHESP - École des Hautes Études en Santé Publique [EHESP]
11 Irset - Institut de recherche en santé, environnement et travail
12 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
13 Service de Néphrologie [Rouen]
14 INI-CRCT - Cardiovascular and Renal Clinical Trialists [Vandoeuvre-les-Nancy]
15 CESP - Centre de recherche en épidémiologie et santé des populations
16 Hôpital Ambroise Paré [AP-HP]
17 Cardiovascular & Renal Clinical Trialists - CRCT - French-Clinical Research Infrastructure Network - F-CRIN [Paris]
18 UVSQ - Université de Versailles Saint-Quentin-en-Yvelines
19 CHU Reims - Hôpital universitaire Robert Debré [Reims]
20 BIOSPECT - Biospectroscopie Translationnelle - EA 7506
21 UGA UFRM - Université Grenoble Alpes - UFR Médecine
22 Université de Poitiers - Faculté de Médecine et de Pharmacie
23 CHU de Poitiers [La Milétrie] - Centre hospitalier universitaire de Poitiers = Poitiers University Hospital
24 MP3CV - Mécanismes physiopathologiques et conséquences des calcifications vasculaires - UR UPJV 7517
25 Service de Néphrologie-Dialyse-Transplantation [CHU Amiens-Picardie]
Dimitri Titeca-Beauport

Résumé

BACKGROUND: Glomerulonephritis (GN) with non-Randall-type, non-cryoglobulinaemic monoclonal immunoglobulin G deposits encompasses rare diseases [proliferative GN with non-organized deposits (PGNMID) and immunotactoid GN] that cannot be distinguished without ultrastructural analysis by electron microscopy (EM). METHODS: Here, we report and analyse the prognosis of 41 EM-proven (PGNMID for 39/41) and 22 non-EM-proven/DNAJB9-negative cases, diagnosed between 2001 and 2019 in 12 French nephrology centres. RESULTS: Median (interquartile range) serum creatinine (SCr) at presentation was 150 (92-256) \textmu mol/L. The predominant histological pattern was membranoproliferative GN (79%), with IgG3 (74%) kappa (78%) deposits the most frequently observed. Disease presentation and patient management were similar between EM-proven and non-EM-proven cases. A serum monoclonal spike was detected for 21 patients and 10 had an underlying haematological malignancy. First-line therapy was mixed between clone-targeted therapy (n = 33), corticosteroids (n = 9) and RAAS inhibitors (n = 19). After 6 months, nine patients achieved complete and 23 partial renal recovery. In univariate analysis, renal recovery was associated with baseline SCr (odds ratio 0.70, P = 0.07). After a median follow-up of 52 (35-74) months, 38% of patients had progressed to end-stage kidney disease independently associated with baseline SCr [hazard ratio (HR) 1.41, P = 0.003] and glomerular crescentic proliferation (HR 4.38, P = 0.004). CONCLUSIONS: Our results confirm that non-cryoglobulinaemic and non-Randall GN with monoclonal IgG deposits are rarely associated with haematological malignancy. The prognosis is uncertain but may be improved by early introduction of a specific therapy.
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Dates et versions

hal-03767975 , version 1 (20-01-2023)

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Ophélie Fourdinier, Marc Ulrich, Alexandre Karras, Jérôme Olagne, David Buob, et al.. Glomerulonephritis with Non-Randall-type, Non-Cryoglobulinaemic Monoclonal Immunoglobulin G Deposits (PGNMID and ITG).. Clinical Kidney Journal, 2022, 15 (9), pp.1727--1736. ⟨10.1093/ckj/sfac085⟩. ⟨hal-03767975⟩
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